Specimens of human otoconia obtained from autopsy material and representing various stages from fetal to advanced old age, were studied by microdissection, scanning electron microscopy, electron microprobe analysis, and x-ray powder diffraction. The typical adult otoconial configuration is a cylindrical, finely serrated body with pointed ends; crystallographically, it corresponds to a single crystal of calcite. Other, less numerous typed include jointed otoconia, pure rhombohedrons and multifaceted, presumably immature forms. Many otoconia achieve the adult configuration during fetal development. The multifaceted otoconia are most numerous, and the rhombohedrons proliferate, during childhon in the young adult, but saccular otoconia are the larger. In middle and advanced age the otoconia decrease in number, especially in the saccule. Saccular otoconia degenerate progressively in a posteroanterior direction across the macula; they assume a specific, fibours, hollowed-out appearance, which is not duplicated by either chemical etching or autolysis. Neogenesis and growth of otoconia appear to occur postnatally, with different characteristic growth potentials for those of the saccule and the utricle. Age-related saccular otoconial degeneration appears to involve the organic material, which disappears either before or simultaneously with the mineral substance.
In our temporal bone material, sensory and neural degeneration do not occur independently. Usually there is a good correlation between the extent and severity of hair cell loss and the nerve degeneration in the osseous spiral lamina. Sensorineural degeneration in ototoxicity and after sound exposure is mostly sensory in nature in the initial phase of the process. Presence of supporting elements effectively delays secondary nerve degeneration. Degeneration of Corti's organ can often be complete, but the corresponding nerve degeneration is usually only severe to subtotal, never complete. Sensorineural degeneration which is predominantly neural (without parallel hair cell loss) is rare and occurred in less than 5% of our material. Severe subtotal nerve degeneration with hair cells still remaining was found only in one single temporal bone. Furthermore, Spoendlin's experiments in the cat suggest that in man also a portion of the first-order cochlear neuron could have an unusual degeneration behavior and may not degenerate in the case of compression or transection. The fact that sensory and neural degeneration rarely occur in reasonably “pure” forms may explain why it is often difficult to distinguish between them by means of audiological tests alone.
The melanocytes of the vestibular labyrinth as seen in colored guinea pigs show a characteristic pattern of distribution in the wall of the utricle and in the ampullae, but they are not present in the wall of the saccule. They are found mainly in well-vascularized regions of apparent secretory or metabolic importance, including the "dark cell" areas. Their dendrite-like processes are often in intimate contact with the capillaries, although no pinocytotic vesicles or other indications of transfer of substances between the melanocytes and the capillary endothelium are seen under the electron microscope. In the human ear, the apparent density of the melanocytes varies with skin color. They are numerous in the cochlea, especially in the bony wall of the modiolus and on the osseous spiral lamina, and they occur also in Reissner's membrane and in the stria vascularis. In the vestibular system they are found in the wall of the saccule as well as in the utricle, the crus commune, and the ampullae, but not in the semicircular canals. They tend to be diffusely scattered, rather than to fonn the well-defined, intensely pigmented areas that are characteristic of the guinea pig. Close contacts with capillaries are seen mainly in the tympanic portion of the spiral ligament. The significance of melanin and the melanocytes in the labyrinth is unknown, but both the anatomical relation of certain melanocytes to capillaries, and the biocheInical relation of melanin to the catecholamines support the hypothesis that they may have a vasomotor function. Other evidence, including the not infrequent association of sensorineural deafness with hereditary disorders of pigmentation also suggests that the melanocytes may play a role of some biological consequence in the inner ear.
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